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A Practical Approach to Vertigo: A Synthesis of the Emerging Evidence

Article  in  Internal Medicine Journal · August 2020

DOI: 10.1111/imj.15013

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 Title: A Practical Approach to Vertigo: A Synthesis of the Emerging Evidence

1. Warwick Rivlin, Medical Registrar, Sunshine Coast University Hospital, QLD, Australia
warwick.rivlin@health.qld.gov.au

2. Courtney Habershon, Medical Registrar, Sunshine Coast University Hospital, QLD, Australia
Courtney.habershon@gmail.com

3. Benjamin Kwok-Tung Tsang, Neurologist, Sunshine Coast University Hospital, QLD, Australia
bktsang@me.com

4. Diego Kaski, Neurologist, University College London Hospital, London, United Kingdom
d.kaski@ucl.ac.uk

Vestibular presentations represent a large financial and symptomatic burden of disease1,2, while remaining one of the
most elusive presentations to accurately and confidently diagnose. A primary cause for this is that the same symptom
can be the end-product of numerous aetiologies, and uncertainties can lead to unnecessary investigations and
associated increased cost and delays in diagnosis. An effective method to narrow the diagnosis is firstly to determine,
from a limited list, which type of vestibular syndrome the patient possesses, and then apply a focussed history and
examination to define the most likely aetiology within that syndrome. This review provides a diagnostic approach to
the vertiginous patient, outlining the underlying pathophysiology that accounts for the clinical symptoms and signs.
With this approach, physicians should be able to diagnose the majority of common vestibular presentations and know
when to refer the urgent, complex, or rare cases to sub-specialist neuro-otologists for prompt and appropriate
management.

What’s in a Name?

In this review, focus will be on the term ‘vertigo’, defined as the perception of falsely present or abnormal self-motion,
as almost all acute vestibular disorders will present with this symptom. Distinction between variant descriptors such
as “dizziness”, “imbalance” or more ambiguous accounts is rarely of diagnostic utility, with many patients reporting
vague or even self-contradictory descriptions3. Instead, a focus on more objective measures such as timing and triggers
is often more useful. Thus, a clear description of “light-headedness” triggered by postural change (with respect to
gravity) and without head positional change, is likely due to orthostatic hypotension.

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version and the Version of Record. Please cite this article as doi: 10.1111/imj.15013

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A Practical Approach to Vertigo: A Synthesis of Emerging Evidence

Abstract:

Vestibular presentations are common in both the acute and recurrent setting, burdening emergency departments
and community clinics alike. Commonly, an unease among the emergency or general physician is felt, and historically
focus has been on gaining knowledge of each potential disease rather than honing the diagnostic process.
Consequently, this paper focuses on the approach itself, helping to categorise this common complaint into one of
four main syndromes: the Acute Vestibular Syndrome, Recurrent Positional Vertigo, Recurrent Spontaneous Vertigo,
and Imbalance. Its simplicity is aimed to minimise uncertainty and highlight clear scenarios when to refer. Together
with descriptions of the clinically relevant pathophysiology, the reader should approach the vertiginous patient with
a new clarity.
Dizziness: A Diagnostic Overview
AVS / ATVS
A limited number of vestibular syndromes and Recurrent Spontaneous • Posterior Circulation
underlying conditions cause the majority of Vertigo Stroke
patients’ vestibular symptoms. Four common • Ménière’s Disease • Vestibular Neuritis /
vestibular syndromes and their frequently • Vestibular Migraine labyrinthitis
associated aetiologies are outlined in Table 1, and
comprehensively discussed by Welgampola et al4.
With this in mind, prioritising the recognition and
Recurrent Positional Imbalance
treatment of urgent conditions, chiefly stroke, is
Vertigo • Vestibular Hypofunction
imperative. As such, the key red flag symptom
• BPPV • Ataxias
would be the first-ever attack of acute o Cerebellar / Sensory
• Central Positional
spontaneous vertigo – the Acute Vestibular
Vertigo
Syndrome – which should yield a low threshold to
refer for urgent assessment given concerns for a Table 1: The key vestibular syndromes, and most common conditions. AVS = acute
vestibular syndrome; ATVS = acute transient vestibular syndrome, BPPV = benign
posterior circulation stroke5,6. If the vertiginous paroxysmal positional vertigo
presentation is recurrent in nature, the aetiology is
more commonly benign, albeit debilitating. Recurrent vertigo can be further categorised by the presence or absence
of positional provocation. Recurrent Positional Vertigo is often caused by benign paroxysmal positional vertigo (BPPV),
while Recurrent Spontaneous Vertigo is often secondary to vestibular migraine or Ménière’s disease. Finally, a
presentation of Generalised Imbalance often points towards vestibular hypofunction (especially bilateral
hypofunction) or ataxia. For those with chronic or recurrent symptoms, a change in pattern should prompt
consideration of a new pathology, rather than simply an atypical presentation of a recurrent condition.

Syndrome 1 – The Acute Vestibular Syndrome

While many conditions may lead to permanent disability over

months or years, a disruption to the posterior circulation may cause
immediate irreparable damage. The first ever attack of acute
spontaneous vertigo may represent a Posterior Circulation Stroke
(PCS), especially in those with cardiovascular risk factors. When
symptoms are present for more than 24 hours, it is termed the Acute
Vestibular Syndrome (AVS), or if lasting less than 24 hours, Acute
Transient Vestibular Syndrome (ATVS). Despite this categorisation, a
short duration of symptoms should not be inferred as low risk for an
ischaemic cause, as PCS can frequently present with symptoms
lasting only minutes5. Associated symptoms can include nausea,
vomiting, autonomic symptoms (eg. palpitations, diaphoresis) and
head motion intolerance6, although these are not specific to PCS. Figure 1 – Vestibulo-Ocular Reflex (VOR) viewed from
above. A left head turn results in opposite perilymphatic
flow, resulting in right gaze shift.

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A PCS can be effectively and rapidly differentiated from an acute Vestibular Neuritis (the two most common aetiologies
of AVS) by using the abbreviated bedside examination assessing vestibular pathways and eye movements, the HINTS
test7. It’s sensitivity out-performs even early MRI scanning7,8, though this is highly dependent on technical
competence. Full methodology of the exam can be reviewed in its original paper7; briefly summarised, it assesses for
the following components:

• Head Impulse Test (HIT) – a brief but rapid 10-15o lateral head turn9.
• Nystagmus.
• Test of Skew - for ocular misalignment on alternate eye cover testing.

The “HINTS plus” test screens for additional hearing loss using bedside finger
rub next to the patient’s ear of the suspected lesional side.

In PCS, specific features may reveal:

• The absence of a catch-up saccade on HIT (i.e. similar to an unaffected

individual, eyes will remain fixated).
• Central nystagmus patterns10, including (but not limited to):
o Spontaneous vertical.
o Pure torsional.
o Direction-changing - where the fast beat changes towards the
direction of gaze (i.e right-beating on right gaze, left-beating
on left gaze).
• Skew deviation (a vertical misalignment of the eyes).

This is deemed a “central” or “dangerous” HINTS exam. While skew can be

Figure 2 - Orientation of the semicircular canals
rarely caused by a peripheral pathology11, direction-changing nystagmus is
highly specific for a central disorder.

A common benign cause for AVS/ATVS is inflammation to either the vestibular branch of the vestibulocochlear nerve,
causing Vestibular Neuritis (VN), or the labyrinth as a whole, Labyrinthitis, the latter leading to additional hearing loss
and being far less common. A preceding or concurrent infectious illness, while supportive of the diagnosis, is present
in under half of cases12. Here, examination typically reveals:

• A delayed catch-up saccade (seen on HIT towards the lesional side).

• Nystagmus is often unidirectional, fast-beating away from the lesion, enhanced on gaze away from the
affected ear.
• Skew deviation is typically absent (though if present will be associated with unidirectional nystagmus)11.

In this case, where all features suggest peripheral aetiology the HINTS-plus result is deemed “benign”. The term “HINTS
negative” is ambiguous, as it may imply negative findings on all aspects of the test including HIT, which in fact suggests
central pathology. An important, though rare, mimic of VN is a PCS of the internal auditory artery, usually a branch of
the anterior inferior cerebellar artery (AICA). This leads to a vascular, rather than inflammatory, hypofunction of the
labyrinth, which may lead to presentations indistinguishable from labyrinthitis. Therefore, any acute unilateral hearing
impairment, particularly in the presence of acute vertigo, should be treated as an AICA stroke until proven otherwise.

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Associated localising neurological signs in PCS such as ataxia (unilateral limb or truncal), intention tremor or dysarthria
are present in only 51% of cases7, hence while useful when present should not be deemed an exclusionary negative
finding. Associated deficits in cranial nerves may also be present in PCS, caused by hypoperfusion of the corresponding
brainstem nuclei. In the young patient, vertebral artery dissection is a particularly common cause of PCS13, and of these
approximately two thirds present with either neck pains or headache, with highly variable frequencies of
aforementioned posterior circulation features14. While PCS and VN represent most AVS presentations, a first
presentation of a recurrent vestibular syndrome such as Vestibular Migraine or Ménière’s Disease is possible
(described later).

Syndrome 2 – The Recurrent Positional Vertigo Syndrome

Recurrent positional vertigo is most often caused by BPPV, which

accounts for an estimated 12% of neurology presentations to the
Emergency Department15, and make up at least 20% of dedicated dizzy
clinic encounters16,17. Within the semicircular canals (SCC), BPPV involves
dislodged otoconia from the otolith organs which pathologically displace
the cupula (Figure 3). Owing to its inferior position, the posterior canal Figure 3 – Otoconia as canalolithiasis and cupulolithiasis,
displacing the cupula in opposite directions
accounts for most BPPV, with lateral (10-20%), anterior (5%), and multiple
canals involved rarely18. Patients suffer unsteadiness or vertigo triggered by head movements such as rolling in bed or
reaching for items from the floor or high shelves. Diagnosis requires both symptoms and nystagmus of the
corresponding canal on canal testing19.

Dislodged otoconia most commonly manifest as canalolithiasis, in which otoconia freely move in the canal, and less
commonly as cupulolithiasis, where they are adhered to the cupula. While descriptions here allude to the former by
default, the latter may result in atypical and sometimes opposite findings (Figure 3). When diagnosing BPPV,
nystagmus will usually start with a latency of approximately 2-20s after the head touches the couch on positional test
and last for less than a minute with a crescendo-decrescendo pattern. Other specific (although not necessarily
sensitive) features supporting BPPV include a reversal of the nystagmus direction when returning to the upright
position, and fatiguability of the nystagmus with repeated testing20.

The Dix-Hallpike (DH) test for diagnosis will yield the following:

• Posterior SCC (canalolithiasis): Up-beating & torsional nystagmus, with the superior aspect of the eyes rotating
towards the ground (geotropic)18. As the DH position is included in the corresponding Epley manoeuvre,
treatment can be conveniently carried out by completing the therapeutic sequence of movements18. Adequate
neck movements are needed for this manoeuvre, therefore limitations in either the patient’s neck mobility or
the consult room may necessitate an alternate repositioning therapy, the Semont manoeuvre.
• Lateral SCC BPPV (canalolithiasis): Lateral nystagmus, (geotropic), confirmed on a lateral roll towards the
direction of nystagmus, and is treated with a Barbecue Roll18. Nystagmus away from the ground (apogeotropic)
may be from contralateral SCC canalolithiasis or ipsilateral cupulolithiasis – the latter often being more
refractory to repositioning manoeuvres.
• Anterior SCC BPPV (canalolithiasis): Manifests as down-beating nystagmus, however this is considerably rare
and should be referred to a specialist centre.

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Full descriptions of canal testing and their therapeutic manoeuvres are described elsewhere18. Atypical findings which
should lower referral threshold include dubious positional provocation, or nystagmus that is prolonged (>60s), without
a latency period, purely vertical (without a torsional component), not fitting a classical pattern for the performed
manoeuvre or consistent with multiple canal involvement. Disproportionate vomiting or associated posterior
circulation features described earlier may indicate either an alternate or additional diagnosis. These features raise
concern for Central Positional Vertigo, an umbrella term describing positional vertigo due to central pathologies. These
include space occupying lesions, previous stroke, demyelination, congenital, neurodegenerative and numerous other
causes21.

Syndrome 3 – The Recurrent Spontaneous Vertigo Syndrome

While the first-ever bout of spontaneous vertigo raises concerns for vascular aetiology, Recurrent Spontaneous Vertigo
is most often caused by either Vestibular Migraine or Ménière’s disease. When experienced for the first time, these
conditions will present as an AVS, and despite their distinct characteristics and associated clinical features they can
still cause diagnostic difficulties even for the seasoned neuro-otologist, especially if presenting as isolated vertigo.

Vestibular Migraine

Vestibular migraine (VM) is a recently recognised entity accounting for approximately 7-10% of dizzy clinic
referrals22,23, though its recognition has increased following large epidemiological studies. Clinical diagnosis requires
recurrent:

• Attacks of spontaneous vestibular symptoms

• Migrainous features; involving either headache with migrainous qualities, visual aura, or associated features
such as photophobia and phonophobia

Up-to-date diagnostic criteria are outlined by the International Headache Society24. Features include a frustratingly
diverse range of vestibulocochlear findings which may masquerade as alternative diagnoses, but a pre-existing
headache history, background of motion sickness, or associated phono-, phono-, or osmo-phobia with attacks are all
highly suggestive of VM. Other less common inter-ictal associations include tinnitus (monaural or binaural), nystagmus
(spontaneous/positional, gaze evoked or unidirectional), and even occasionally deafness or unilateral aural fullness.
While rarely present, these unilateral auditory features can mimic Ménière’s disease, however audiological testing
helps to distinguish the two (described later) and is usually normal in VM. Notably, examination between attacks is
often normal. Treatment is described elsewhere, but is largely based on preventative therapy as for cephalgic
migraine25,26.

Ménière’s Disease:

Ménière’s Disease (MD) involves idiopathic excess of endolymph within the acoustic apparatus, either by excessive
production or reduced elimination.

Clinically, it involves the triad of:

• Vertigo lasting 20 minutes to 12 hours.

• Audiometrically documented sensorineural hearing loss (predominantly low-frequency).
• Fluctuating aural symptoms (tinnitus or aural fullness).

Specific diagnostic criteria are specified elsewhere27.

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The pathological changes within the labyrinth account for a progressive change in character of nystagmus, and makes
the diagnosis of MD difficult even for otologists. Owing to abnormal fluid shifts within the ear, vestibular neurons are
first stimulated, later inhibited and finally toxified. Clinically, this can manifest as nystagmus which may reverse over
minutes to hours28,29. When present, this tends to be high velocity and horizontal, in contrast to VM causing either
slower horizontal, or vertical nystagmus30. While nystagmus progression is usually completed by 24-48 hours,
symptoms usually spontaneously occur and remit within 12 hours30. If long-standing or severe, permanent vestibular
loss may be present, manifesting as a positive head impulse test.

Vestibular loss can be objectively measured with vestibular investigations such as caloric testing, which if present will
demonstrate reduced caloric responses, or video HIT (vHIT) (Figure 4) exhibiting reduced vestibulo-ocular reflexes or
abnormal catch up saccades. A dissociation between vHIT and caloric testing is suggestive of MD over VM31,32.
Audiometry, revealing characteristic low-to-medium-frequency hearing loss, is needed to formalise the diagnosis, and
may be used serially to screen for permanent loss. However, acute deafness may be absent between bouts if not
measured shortly after onset of an attack, hence its utility is heavily dependent on the timing relative to an attack.
Importantly, owing to MD’s prevalence in the elderly33, a degree of presbycusis (with characteristic high frequency
loss) may be overlaid giving the appearance of pan-frequency loss. In these cases, comparison with the unaffected ear
can highlight the asymmetrical low-frequency loss, unless bilateral MD is present, which can occur particularly in those
with long disease duration34,35. Further diagnostic tests, such as electrocochleography36,37, and treatments are
described elsewhere38.

While outside the scope of this paper, a few remaining rare but notable conditions may contribute to recurrent
spontaneous vertigo. Superior canal dehiscence syndrome (SCDS), is a rare disease involving a third window to the
inner ear through the region of the dehisced superior SCC. This can cause spontaneous vertigo, as well as sound or
pressure-induced vertigo by energy transmission into the vestibular apparatus through the dehisced area. It may
similarly cause autophony, the abnormally loud hearing of one’s own voice or body movements. Diagnosis requires
radiological confirmation, and further details of the condition are comprehensively discussed by Ward and
colleagues.39. Epileptic Vertigo may cause recurrent short-lived spontaneous vertigo, and is most commonly associated
with a temporal lobe focus, although this rarely manifests as vertigo in isolation40. While sometimes associated with
positional changes, Vestibular Paroxysmia classically causes recurrent spontaneous vertigo from vascular compression
of the eighth cranial nerve41. It may be triggered by exercise or heat, is associated with “staccato” or “type-writer”
tinnitus, and is often responsive to carbamazepine or oxcarbazepine42,43.

Syndrome 4 - Imbalance

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Imbalance is the final common vestibular syndrome and can be caused by
numerous conditions spanning across systems.

For instance, if the patient is in fact describing imbalance due to limb

incoordination, consideration of sensory ataxia due to a peripheral
neuropathy, or cerebellar ataxia due to cerebellar pathology is warranted.
Numerous musculoskeletal conditions may result in imbalance, either
through disabling pain or weakness, and its underlying cause should be
addressed. If the imbalance is due to vertigo, the three previously-described
syndromes of AVS, Recurrent Positional Vertigo, and Recurrent Spontaneous
Vertigo should be reviewed. In the absence of these descriptions, Vestibular
Hypofunction (otherwise known as insufficiency or failure) should be
considered. Counterintuitively, chronic vestibular hypofunction rarely
causes vertigo, as vertiginous perception is due to asymmetrical stimulation
from each vestibular centre. If present, the contralateral side compensates
its firing rate within weeks44, alleviating the unpleasant symptom. Hence if
unilateral hypofunction occurs sufficiently slowly, contralateral
compensation occurs at a rate that precludes symptoms.

Vestibular Hypofunction:

Chronic bilateral vestibular hypofunction (BVH), or sufficiently severe

Figure 4 – vHIT showing severely reduced left
unilateral vestibular hypofunction (UVH), can manifest as imbalance. horizontal SCC VOR gain, with compensatory saccades.

BVH is most commonly secondary to:

• Aminoglycoside toxicity, bilateral Ménière’s, trauma and previous intracranial infection, however is still
idiopathic in approximately 50% of cases45-47.

UVH is often secondary to:

• Vestibular neuritis, Ménière’s, schwannomas, or trauma.

Historically, patients will complain of imbalance exacerbated by, or limited to, walking on unfirm or uneven surfaces.
The differential diagnosis for this includes both cerebellar and proprioceptive loss, however vestibular hypofunction
should not exhibit focal sensory or cerebellar deficits on limb examination. Additionally, vestibular hypofunction will
cause visual shaking or wobbling when walking (oscillopsia), particularly on hard surfaces. This is due to reduced
vestibulo-ocular reflexes (VOR) failing to compensate for the transmitted vibrations from walking (Figure 1).
Examination will yield:

• A positive head impulse test (unilaterally in UVH or bilaterally in BVH).

• A decline of at least 3 lines on Snellen chart during dynamic visual acuity test – which assesses vision during
alternating lateral head turns at 2 hertz.
• A marked exacerbation on Romberg’s test whilst on a soft mat48.

Further investigations can objectify vestibular loss, such as the video HIT (Figure 4) and reduced responses on caloric
testing. Treatment involves mitigating further damage by the primary insult, and referral for vestibular rehabilitation
to focus on patients’ remaining proprioceptive and visual inputs, and improve their VOR gain.

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Persistent Postural-Perceptual Dizziness:

A recently renamed entity, persistent postural-perceptual dizziness (PPPD) describes a functional (psychogenic)
continuous dizziness syndrome. The pathophysiology is theorised to be secondary to an initial vestibular insult leading
to maladaptive balance strategies, avoidance behaviour and various psychiatric phenomena49,50. Symptoms are
worsened by complex visual stimulation such as traffic, busy walkways or even movements relative to patterned
surfaces, such as carpets. As a functional neurological disorder, PPPD requires necessary investigations to rule out
other potential causes and may (but not necessarily) include neurological imaging or vestibular function tests. Multi-
modality treatment is effective in this condition and focusses on physiotherapy, cognitive behavioural therapy and
exposure therapy to provoking agents as well as pharmacotherapy, outlined in detail by Popkirov et al49.

Conclusion:

The presentation of vertigo can be challenging, due to its variable description and accompanying features. However,
the delineation of its likely cause can be reliably and simply done by first determining whether it is the first-ever or
recurrent episode. The former should, in almost all instances, be urgently assessed at a tertiary centre for concerns
of a PCS. Recurrent vertigo can be categorised into either spontaneous versus head-positionally triggered. Here, its
detailed features most commonly yield a diagnosis of either BPPV, vestibular migraine, or Ménière’s. Finally, a
presentation of imbalance, is often secondary to vestibular hypofunction or PPPD. This approach, that emphasises a
targeted history and examination rather than radiological investigations, will assist with streamlined referrals and
treatment to minimise the burden of disease in the dizzy patient.

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 History Examination Provisional Dx Investigations / Management
1) Clarify Acuity of Vertigo
Goal: To rapidly screen for time-critical PCS
Acute Vestibular Syndrome
High Risk Features: HI/N/TS/Plus PCS Urgent Referral for stroke
- Cardiovascular risk factors - / Central nystagmus* / + / + workup and management
- Cerebellar or cranial nerve Posterior circulation features
deficits Cerebellar or cranial nerve signs
Low Risk Features HI/N/TS/Plus VN / Confirmation with vHIT
- Absence of high-risk features + / Peripheral nystagmus* / - / + Labyrinthitis
- Preceding viral prodrome (if deafness, consider PCS)
Differentials: First presentation of recurrent vertigo (below), other central pathology such as demyelination or vasculitis
2) Determine Provocation
Goal: Determine provisional diagnosis by targeted history at the three common conditions
Recurrent Positional Vertigo
Positional paroxysms of vertigo Positional testing (eg Dix-Hallpike) BPPV +/- Confirmation with Frenzel
Less than 1 minute Nystagmus corresponding with goggles
canal, latency, reversal of Treat with repositioning
nystagmus on sitting manoeuvres
Refer if atypical
Differentials: Central Positional Vertigo (multiple aetiologies)
Recurrent Spontaneous Vertigo
Unilateral auditory symptoms Nystagmus Meniérè’s Formal audiogram and
(fullness, tinnitus, deafness) Sensorineural hearing loss vestibular testing

Migrainous headache Often normal between attacks Vestibular Review Barany diagnostic
Confirm duration / temporal Migraine criteria
relation to vertigo
Rare Differentials: Vestibular Paroxysmia, Superior Canal Dehiscence Syndrome, Epileptic Vertigo
3) Clarify Imbalance (if present)
Goal: To screen for cerebellar and sensory ataxia, and vestibular hypofunction
Imbalance
Review for ataxia, peripheral Cerebellar exam Ataxia a) Brain imaging
neuropathy & cerebellar symptoms Sensory examination a) Cerebellar b) Nerve conduction studies,
b) Sensory metabolic screen
Oscillopsia HIT positive Vestibular Formalised vestibular testing
Generalised imbalance Reduced dynamic visual acuity Hypofunction (vHIT, Caloric test)
Romberg’s on foam pad
Differentials: PPPD, Extrapyramidal Movement Disorders, CANVAS
Table 2- Diagnostic Table
*Central and peripheral nystagmus patterns described in “Acute Vestibular Syndrome” section. PCS = Posterior Circulation Stroke,
VN = Vestibular Neuritis, BPPV = Benign Paroxysmal Positional Vertigo, vHIT = video head impulse test

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